322.

DISORDERS OF COAGULATION OR FIBRINOLYSIS | NOVEMBER 29, 2018

Transplacental Hemophilia a and Prophylactic Treatment

with Recombinant FVIIa in the Newborn Period
*,1 *,2 *,2 *,3
Selin Aytac, Tolga Celik, Sule Yigit, Fatma Gumruk, MD
1
Hacettepe University Faculty of Medicine, Ankara, Turkey
2
Hacettepe University Faculty of medicine, department of neonatalogy, ankara, Turkey
3
Department of Pediatric Hematology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

Blood (2018) 132 (Suppl_1) : 5024.

http://doi.org/10.1182/blood-2018-99-119760

Abstract
Acquired hemophilia due to inhibitor antibodies is a rare entity during childhood period. Moreover,
transplacental hemophilia during the newborn period as a result of maternal IgG antibodies (inhibitors)
which may cross the placenta into the foetal circulation is very rare and may cause life- threathening
neonatal hemorrhages. The occurence of acquired hemophilia during or after delivery may lead bleeding
tendencies in both the mother and baby.

Here, we want to decribe a newborn baby who delivered from a mother with an initial diagnosis of
acquired Hemophilia A and succesfully treated with a prohylactic rFVIIa.

Case : A 27 years old women who was diagnosed with an acquired hemophilia A after her first delivery
was admitted to our hospital for the second delivery.Her pregnancies were 15 months apart. Her FVIII
level was found to be low (<1%) with a very high titer (>100NBU) inhibitor.She was treated with rFVIIa
before C/S by adult hematologist and after giving a birth to a term boy without any bleeding complication,
newborn baby was referred to our department because of having low FVIII level (0.3 %) with high titer
inhibitor(320 NBU) and an abnormal aPTT mixing test on the first day of life. Cranial and abdominal USG
was found to be normal and despite having no bleeding event we decide to give IVIG treatment due to
very high titer of inhibitor level. On the 3rd day of life some blood in the stool was detected however there
is no decrease in the Hb level. Prophylactic rFVIIa with a dose of 90µg/kg/daily was given during the
next 4 weeks and inhibitor level was checked regulary. His inhibitor level was decraesed gradually on the
first week 256NBU , second week 51 NBU and 4th week 2NBU with a FVIII level 2.7% and propylactic
treatment was stopped on the 5th week of life. Although we did not able to detect inhibitor antibodies by
trying to do aptt mixing test in the breastmilk, his mother did not have enough breatmilk and baby was
supported by infant formula as well.

There was a few numbers of cases with neonatal acquired hemophilia due to transplacental transfer of
autoantibodies reported in the literature . It was reported that most inhibitors spntaneously disappear
over a median 30 months after delivery and FVIII inhibitors are rarely present during labor and delivery
in the next pregnancy. However, in our case 15 months after the first pregnacy he was delivered. The
outcome of the transplasental acquired hemophilia cases reported in the literature was favorable with a
resolution after a median of 3 months (range 0.2-3 months) despite one of them experienced intracranial
hemorrhage.. Although peak inhibitor titer was reported to be 150BUin the literature, in our case it
was the higher than the reported level (320NBU). However despite a very high peak inhibitor titer, we
can speculate that due to limited intake of breastmilk which may also contain inhibitor antibodies may
ameoliorate the prompt decrase of inhibitor antibodies. rFVIIa is safe and protect our patient from life-
threatening bleeding events without any complication.

Disclosures

No relevant conflicts of interest to declare.

Author notes
* Asterisk with author names denotes non-ASH members.

© 2018 by the American Society of Hematology